Diagnostic algorithm for pulmonary arterial hypertension updated at the Fifth World Symposium on Pulmonary Hypertension - Flowchart
Diagnostic algorithm for pulmonary arterial hypertension updated at the Fifth World Symposium on Pulmonary Hypertension - Flowchart Pulmonary Hypertension Pulmonary Hypertension
«Flowchart»

Symptoms, signs, history suggestive of PH

Symptoms, signs, history suggestive of PH

Symptoms, signs, history suggestive of PH

Echocardiography compatible with PH?

Echocardiography compatible with PH?

Echocardiography compatible with PH?

PH unlikely

PH unlikely

PH unlikely

Consider other causes or recheck

Consider other causes or recheck

Consider other causes or recheck

Consider most common causes of PH (i.e, left heart disease, lung disease)

Consider most common causes of PH (i.e, left heart disease, lung disease)

Consider most common causes of PH (i.e, left heart disease, lung disease)

History, signs, risk factors, ECG, X-ray, PFT incl. DLCO, consider BGA, HR-CT

History, signs, risk factors, ECG, X-ray, PFT incl. DLCO, consider BGA, HR-CT

History, signs, risk factors, ECG, X-ray, PFT incl. DLCO, consider BGA, HR-CT

Yes

Yes

Yes

No

No

No

No signs of severe PH/RV dysfunction

No signs of severe PH/RV dysfunction

No signs of severe PH/RV dysfunction

Signs of severe PH/RV dysfunction

Signs of severe PH/RV dysfunction

Signs of severe PH/RV dysfunction

No signs of severe PH/RV dysfunction

No signs of severe PH/RV dysfunction

No signs of severe PH/RV dysfunction

Treat underlying disease

Treat underlying disease

Treat underlying disease

Signs of severe PH/RV dysfunction

Signs of severe PH/RV dysfunction

Signs of severe PH/RV dysfunction

Refer to PH expert center

Refer to PH expert center

Refer to PH expert center

Yes

Yes

Yes

No

No

No

CTEPH likely CT angiography, RHC plus PA (PEA expert center)

CTEPH likely CT angiography, RHC plus PA (PEA expert center)

CTEPH likely CT angiography, RHC plus PA (PEA expert center)

RHC PAPm 25 mmHg, PAWP 15 mmHg, PVR > 3 WU

RHC PAPm 25 mmHg, PAWP 15 mmHg, PVR > 3 WU

RHC PAPm 25 mmHg, PAWP 15 mmHg, PVR > 3 WU

Yes

Yes

Yes

No

No

No

PAH likely Specific diagnostic tests

PAH likely Specific diagnostic tests

PAH likely Specific diagnostic tests

Consider other causes

Consider other causes

Consider other causes

CTD

CTD

CTD

Drugs Toxins

Drugs Toxins

Drugs Toxins

HIV

HIV

HIV

PVOD PCH

PVOD PCH

PVOD PCH

CHD

CHD

CHD

Porto- pulmonary

Porto- pulmonary

Porto- pulmonary

Schistosomiasis

Schistosomiasis

Schistosomiasis

Other (group 5?)

Other (group 5?)

Other (group 5?)

Idiopathic or Heritable PAH

Idiopathic or Heritable PAH

Idiopathic or Heritable PAH

Family history; consider genetic studies (expert centers only)

Family history; consider genetic studies (expert centers only)

Family history; consider genetic studies (expert centers only)

Echocardiography compatible with PH?

Echocardiography compatible with PH?

Echocardiography compatible with PH?

Yes

Yes

Yes

No

No

No

Diagnosis of heart disease or lung disease confirmed?

Diagnosis of heart disease or lung disease confirmed?

Diagnosis of heart disease or lung disease confirmed?

Signs of severe PH/RV dysfunction

Signs of severe PH/RV dysfunction

Signs of severe PH/RV dysfunction

V/Q scintigraphy unmatched perfusion defects?

V/Q scintigraphy unmatched perfusion defects?

V/Q scintigraphy unmatched perfusion defects?

No signs of severe PH/RV dysfunction

No signs of severe PH/RV dysfunction

No signs of severe PH/RV dysfunction

Yes

Yes

Yes

No

No

No

Yes

Yes

Yes

Treat underlying disease

Treat underlying disease

Treat underlying disease

RHC PAPm 25 mmHg, PAWP 15 mmHg, PVR > 3 WU

RHC PAPm 25 mmHg, PAWP 15 mmHg, PVR > 3 WU

RHC PAPm 25 mmHg, PAWP 15 mmHg, PVR > 3 WU

CTEPH likely
CT angiography, RHC plus PA
(PEA expert center)

CTEPH likely
CT angiography, RHC plus PA
(PEA expert center)

CTEPH likely
CT angiography, RHC plus PA
(PEA expert center)



Yes

Yes

Yes

No

No

No

Consider other causes

Consider other causes

Consider other causes

PAH likely Specific diagnostic tests

PAH likely Specific diagnostic tests

PAH likely Specific diagnostic tests

Yes

Yes

Yes

No

No

No

END

END

END

Family history; consider genetic studies (expert centers only)

Family history; consider genetic studies (expert centers only)

Family history; consider genetic studies (expert centers only)

END

END

END

END

END

END

CTD CTD Drugs Toxins Drugs Toxins HIV HIV PVOD PCH PVOD PCH Idiopathic or Heritable PAH Idiopathic or Heritable PAH CHD CHD Porto pulmonary Porto pulmonary Schistosomiasis Schistosomiasis Other (group 5?) Other (group 5?)

END

END

END

Refer to PH expert center

Refer to PH expert center

Refer to PH expert center